Cholangiocarcinomas (CCs) are malignancies of the biliary duct system that may originate in the liver and extrahepatic bile ducts, which terminate at the ampulla of Vater (see, e.g., Douglass H O, et al., In: Holland J F, et al, eds. Cancer Medicine. Vol 2. Philadelphia, Pa.: Lea & Febiger. 1993:1455-62; Lake J R. B In: Sleisinger M H, Fordtran J S, eds. Gastrointestinal Disease. 5th ed. Vol 2. Philadelphia, Pa.: WB Saunders. 1993:1891-1902; Lotze M T, et al., In: Devita V, Hellman S, Rosenberg S. Cancer: Principles and Practice of Oncology. 4th. Philadelphia, Pa.: Lippincott; 1993:883-907; de Groen P C, et al., N Engl J. Med. Oct. 28 1999; 341(18):1368-78). CCs are encountered in 3 geographic regions: intrahepatic, extrahepatic (ie, perihilar), and distal extrahepatic. Perihilar tumors are the most common CCs, and intrahepatic tumors are the least common Perihilar tumors, also called Klatskin tumors occur at the bifurcation of right and left hepatic ducts. Distal extrahepatic tumors are located from the upper border of the pancreas to the ampulla. More than 95% of these tumors are ductal adenocarcinomas; many patients present with unresectable or metastatic disease.
Cholangiocarcinoma is a tumor that arises from the intrahepatic or extrahepatic biliary epithelium. More than 90% are adenocarcinomas, and the remainder are squamous cell tumors. The etiology of most bile duct cancers remains undetermined Long-standing inflammation, as with primary sclerosing cholangitis (PSC) or chronic parasitic infection, has been suggested to play a role by inducing hyperplasia, cellular proliferation, and, ultimately, malignant transformation. Intrahepatic cholangiocarcinoma may be associated with chronic ulcerative colitis and chronic cholecystitis.
Cholangiocarcinomas tend to grow slowly and to infiltrate the walls of the ducts, dissecting along tissue planes. Local extension occurs into the liver, porta hepatis, and regional lymph nodes of the celiac and pancreaticoduodenal chains. Life-threatening infection (cholangitis) may occur that requires immediate antibiotic intervention and aggressive biliary drainage.
Despite aggressive anticancer therapy and interventional supportive care (ie, wall stents or percutaneous biliary drainage), median survival rate is low, since most patients (90%) are not eligible for curative resection. The overall survival is approximately 6 months.
As such, improved methods for early stage detection of cholangiocarcinomas is needed.